Use of 3-Dimensional Volumetric Modeling of Adrenal Gland Size in Patients with Primary Pigmented Nodular Adrenocortical Disease
| Autor: | Georgios Z. Papadakis, C. A. Stratakis, P. P. Chrysostomou, Maya Lodish, Evrim B. Turkbey |
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| Rok vydání: | 2016 |
| Předmět: |
Male
Hydrocortisone Endocrinology Diabetes and Metabolism Clinical Biochemistry 030204 cardiovascular system & hematology Biochemistry Congenital Cushing syndrome 0302 clinical medicine Endocrinology Adrenal Glands Child Cushing Syndrome Body surface area adrenals cushing's syndrome Adrenal gland Organ Size General Medicine Cone-Beam Computed Tomography Middle Aged Hyperplasia Hypercortisolemia medicine.anatomical_structure Child Preschool Biomedical Imaging Female CT medicine.drug Adult medicine.medical_specialty Adolescent Cyclic AMP-Dependent Protein Kinase RIalpha Subunit Clinical Sciences 030209 endocrinology & metabolism Article Endocrinology & Metabolism Young Adult 03 medical and health sciences Rare Diseases Clinical Research 3D imaging Internal medicine medicine Humans Preschool Adrenal Hyperplasia Dexamethasone Adrenal Hyperplasia Congenital business.industry Biochemistry (medical) medicine.disease PPNAD business Primary pigmented nodular adrenocortical disease |
| Zdroj: | Hormone and metabolic research = Hormon-und Stoffwechselforschung = Hormones et metabolisme, vol 48, iss 4 |
| ISSN: | 1439-4286 0018-5043 |
| DOI: | 10.1055/s-0042-103686 |
| Popis: | Primary pigmented nodular adrenocortical disease (PPNAD) is a rare type of bilateral adrenal hyperplasia leading to hypercortisolemia. Adrenal nodularity is often appreciable with computed tomography (CT); however, accurate radiologic characterization of adrenal size in PPNAD has not been studied well. We used 3-dimensional (3D) volumetric analysis to characterize and compare adrenal size in PPNAD patients, with and without Cushing's syndrome (CS). Patients diagnosed with PPNAD and their family members with known mutations in PRKAR1A were screened. CT scans were used to create 3D models of each adrenal. Criteria for biochemical diagnosis of CS included loss of diurnal variation and/or elevated midnight cortisol levels, and paradoxical increase in urinary free cortisol and/or urinary 17-hydroxysteroids after dexamethasone administration. Forty-five patients with PPNAD (24 females, 27.8±17.6 years) and 8 controls (19±3 years) were evaluated. 3D volumetric modeling of adrenal glands was performed in all. Thirty-eight patients out of 45 (84.4%) had CS. Their mean adrenal volume was 8.1 cc±4.1, 7.2 cc±4.5 (p=0.643) for non-CS, and 8.0cc±1.6 for controls. Mean values were corrected for body surface area; 4.7 cc/kg/m(2)±2.2 for CS, and 3.9 cc/kg/m(2)±1.3 for non-CS (p=0.189). Adrenal volume and midnight cortisol in both groups was positively correlated, r=0.35, p=0.03. We conclude that adrenal volume measured by 3D CT in patients with PPNAD and CS was similar to those without CS, confirming empirical CT imaging-based observations. However, the association between adrenal volume and midnight cortisol levels may be used as a marker of who among patients with PPNAD may develop CS, something that routine CT cannot do. |
| Databáze: | OpenAIRE |
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